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Acute phase response

MedGen UID:
7874
Concept ID:
C0001349
Pathologic Function
Synonyms: Acute Phase Reaction; Acute Phase Response; Acute Phase Responses; Acute Phase State; Acute-Phase Reaction; Acute-Phase Response; Acute-Phase State; Phase Response, Acute; Reaction, Acute Phase; Reaction, Acute-Phase; Response, Acute Phase; Response, Acute-Phase; State, Acute-Phase
 
HPO: HP:0033331

Definition

Presence of one or more manifestations of the acute phase response. Acute phase proteins (APP) are blood proteins primarily synthesized by hepatocytes as part of the acute phase response (APR). The APR is part of the early-defense or innate immune system, which is triggered by different stimuli including trauma, infection, stress, neoplasia, and inflammation. The APR results in a complex systemic reaction with the goal of reestablishing homeostasis and promoting healing. [from HPO]

Conditions with this feature

Proteasome-associated autoinflammatory syndrome 5
MedGen UID:
1779962
Concept ID:
C5543027
Disease or Syndrome
Proteasome-associated autoinflammatory syndrome 4
MedGen UID:
1780127
Concept ID:
C5543053
Disease or Syndrome
Proteasome-associated autoinflammatory syndrome-4 (PRAAS4) is an autosomal recessive immunologic disorder characterized by onset of panniculitis and erythematous skin lesions in early infancy. Additional features include hepatosplenomegaly, lymphadenopathy, fever, generalized lipodystrophy, myositis, and joint contractures, as well as delayed motor and speech development. Autoimmune features, such as hemolytic anemia, may also occur. Laboratory studies show elevation of acute phase reactants and abnormal activation of the type I interferon response. Treatment with the JAK (see 147795) inhibitor ruxolitinib may result in clinical improvement (summary by de Jesus et al., 2019). For a discussion of genetic heterogeneity of PRAAS, see PRAAS1 (256040).

Professional guidelines

PubMed

van Riel PL, Renskers L
Clin Exp Rheumatol 2016 Sep-Oct;34(5 Suppl 101):S40-S44. Epub 2016 Oct 18 PMID: 27762189
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G
Arthritis Rheum 2010 Sep;62(9):2569-81. doi: 10.1002/art.27584. PMID: 20872595
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovsky J, Wolfe F, Hawker G
Ann Rheum Dis 2010 Sep;69(9):1580-8. doi: 10.1136/ard.2010.138461. PMID: 20699241

Recent clinical studies

Etiology

Dzobo KE, Kraaijenhof JM, Stroes ESG, Nurmohamed NS, Kroon J
Atherosclerosis 2022 May;349:101-109. doi: 10.1016/j.atherosclerosis.2022.04.004. PMID: 35606070
Deshayes S, Aouba A, Grateau G, Georgin-Lavialle S
Int J Clin Pract 2021 Jun;75(6):e13966. Epub 2021 Jan 20 doi: 10.1111/ijcp.13966. PMID: 33368925
Nairz M, Weiss G
Met Ions Life Sci 2019 Jan 14;19 doi: 10.1515/9783110527872-011. PMID: 30855106
Kasperska-Zajac A
J Eur Acad Dermatol Venereol 2012 Jun;26(6):665-72. Epub 2011 Nov 25 doi: 10.1111/j.1468-3083.2011.04366.x. PMID: 22118494
Eiam-Ong S
Semin Nephrol 2003 Jan;23(1):21-33. doi: 10.1053/snep.2003.50002. PMID: 12563598

Diagnosis

Unver N, McAllister F
Cytokine Growth Factor Rev 2018 Jun;41:10-17. Epub 2018 Apr 18 doi: 10.1016/j.cytogfr.2018.04.004. PMID: 29699936Free PMC Article
van Riel PL, Renskers L
Clin Exp Rheumatol 2016 Sep-Oct;34(5 Suppl 101):S40-S44. Epub 2016 Oct 18 PMID: 27762189
Fliers E, Bianco AC, Langouche L, Boelen A
Lancet Diabetes Endocrinol 2015 Oct;3(10):816-25. Epub 2015 Jun 10 doi: 10.1016/S2213-8587(15)00225-9. PMID: 26071885Free PMC Article
Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC
J Autoimmun 2014 Feb-Mar;48-49:84-9. Epub 2014 Jan 28 doi: 10.1016/j.jaut.2014.01.029. PMID: 24485157
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G
Arthritis Rheum 2010 Sep;62(9):2569-81. doi: 10.1002/art.27584. PMID: 20872595

Therapy

Reid IR, Green JR, Lyles KW, Reid DM, Trechsel U, Hosking DJ, Black DM, Cummings SR, Russell RGG, Eriksen EF
Bone 2020 Aug;137:115390. Epub 2020 Apr 27 doi: 10.1016/j.bone.2020.115390. PMID: 32353565
Bharadwaj U, Kasembeli MM, Robinson P, Tweardy DJ
Pharmacol Rev 2020 Apr;72(2):486-526. doi: 10.1124/pr.119.018440. PMID: 32198236Free PMC Article
Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC
J Autoimmun 2014 Feb-Mar;48-49:84-9. Epub 2014 Jan 28 doi: 10.1016/j.jaut.2014.01.029. PMID: 24485157
Papapetrou PD
Hormones (Athens) 2009 Apr-Jun;8(2):96-110. doi: 10.14310/horm.2002.1226. PMID: 19570737
Eiam-Ong S
Semin Nephrol 2003 Jan;23(1):21-33. doi: 10.1053/snep.2003.50002. PMID: 12563598

Prognosis

Overmyer KA, Shishkova E, Miller IJ, Balnis J, Bernstein MN, Peters-Clarke TM, Meyer JG, Quan Q, Muehlbauer LK, Trujillo EA, He Y, Chopra A, Chieng HC, Tiwari A, Judson MA, Paulson B, Brademan DR, Zhu Y, Serrano LR, Linke V, Drake LA, Adam AP, Schwartz BS, Singer HA, Swanson S, Mosher DF, Stewart R, Coon JJ, Jaitovich A
Cell Syst 2021 Jan 20;12(1):23-40.e7. Epub 2020 Oct 8 doi: 10.1016/j.cels.2020.10.003. PMID: 33096026Free PMC Article
Unver N, McAllister F
Cytokine Growth Factor Rev 2018 Jun;41:10-17. Epub 2018 Apr 18 doi: 10.1016/j.cytogfr.2018.04.004. PMID: 29699936Free PMC Article
van Riel PL, Renskers L
Clin Exp Rheumatol 2016 Sep-Oct;34(5 Suppl 101):S40-S44. Epub 2016 Oct 18 PMID: 27762189
Fliers E, Bianco AC, Langouche L, Boelen A
Lancet Diabetes Endocrinol 2015 Oct;3(10):816-25. Epub 2015 Jun 10 doi: 10.1016/S2213-8587(15)00225-9. PMID: 26071885Free PMC Article
Grassi W, De Angelis R, Lamanna G, Cervini C
Eur J Radiol 1998 May;27 Suppl 1:S18-24. doi: 10.1016/s0720-048x(98)00038-2. PMID: 9652497

Clinical prediction guides

Papafilippou L, Claxton A, Dark P, Kostarelos K, Hadjidemetriou M
Adv Healthc Mater 2021 Jan;10(1):e2001378. Epub 2020 Nov 25 doi: 10.1002/adhm.202001378. PMID: 33236524
Perez L
Arch Biochem Biophys 2019 Aug 15;671:196-202. Epub 2019 Jul 16 doi: 10.1016/j.abb.2019.07.013. PMID: 31323216Free PMC Article
van Riel PL, Renskers L
Clin Exp Rheumatol 2016 Sep-Oct;34(5 Suppl 101):S40-S44. Epub 2016 Oct 18 PMID: 27762189
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G
Arthritis Rheum 2010 Sep;62(9):2569-81. doi: 10.1002/art.27584. PMID: 20872595
Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovsky J, Wolfe F, Hawker G
Ann Rheum Dis 2010 Sep;69(9):1580-8. doi: 10.1136/ard.2010.138461. PMID: 20699241

Recent systematic reviews

Murray HC, Osterman C, Bell P, Vinnell L, Curtis MA
Acta Neuropathol Commun 2022 Aug 6;10(1):108. doi: 10.1186/s40478-022-01413-9. PMID: 35933388Free PMC Article
Yin X, Tian F, Wu B, Xu T
Cochrane Database Syst Rev 2022 Mar 29;3(3):CD010893. doi: 10.1002/14651858.CD010893.pub4. PMID: 35349164Free PMC Article
Silva MC, Furlanetto TW
Nutr Res 2015 Feb;35(2):91-6. Epub 2014 Dec 31 doi: 10.1016/j.nutres.2014.12.008. PMID: 25631715
Rytter MJ, Kolte L, Briend A, Friis H, Christensen VB
PLoS One 2014;9(8):e105017. Epub 2014 Aug 25 doi: 10.1371/journal.pone.0105017. PMID: 25153531Free PMC Article
Visser J, Labadarios D, Blaauw R
Nutrition 2011 Jul-Aug;27(7-8):745-58. doi: 10.1016/j.nut.2010.12.009. PMID: 21679878

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